Warning: imagejpeg(C:\Inetpub\vhosts\kidney.de\httpdocs\phplern\26199027
.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 Can+J+Cardiol
2015 ; 31
(11
): 1313-24
Nephropedia Template TP
gab.com Text
Twit Text FOAVip
Twit Text #
English Wikipedia
Pathogenesis of Arrhythmogenic Cardiomyopathy
#MMPMID26199027
Asimaki A
; Kleber AG
; Saffitz JE
Can J Cardiol
2015[Nov]; 31
(11
): 1313-24
PMID26199027
show ga
Arrhythmogenic cardiomyopathy (ACM) is a primary myocardial disease. It is
characterized by frequent ventricular arrhythmias and increased risk of sudden
cardiac death typically arising as an early manifestation before the onset of
significant myocardial remodelling. Myocardial degeneration, often confined to
the right ventricular free wall, with replacement by fibrofatty scar tissue,
develops in many patients. ACM is a familial disease but genetic penetrance can
be low and disease expression is highly variable. Inflammation might promote
disease progression. It also appears that exercise increases disease penetrance
and accelerates its development. More than 60% of probands harbour mutations in
genes that encode desmosomal proteins, which has raised the possibility that
defective cell-cell adhesion might play a role in disease pathogenesis. Recent
advances have implicated changes in the canonical wingless-type mouse mammary
tumour virus integration site (Wnt)/?-catenin and Hippo signalling pathways and
defects in forwarding trafficking of ion channels and other proteins to the
intercalated disk in cardiac myocytes. In this review we summarize the current
understanding of the pathogenesis of ACM and highlight future research
directions.
|*Genetic Predisposition to Disease
[MESH]
|Arrhythmogenic Right Ventricular Dysplasia/*genetics
[MESH]
free
free
free
